Eichinger Katy, Heatwole Chad, Heininger Susanne, Stinson Nikia, Matichak Stock Carly, Grosmann Carla, Wagner Kathryn R, Tawil Rabi, Statland Jeffrey M
Department of Neurology, University of Rochester Medical Center, Box 673, 601 Elmwood Avenue, Rochester, New York, 14642.
Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
Muscle Nerve. 2017 Mar;55(3):333-337. doi: 10.1002/mus.25251. Epub 2016 Dec 5.
In preparation for future clinical trials, we determined the reliability, relationship to measures of disease severity, and consistency across sites of the 6 Minute Walk Test (6MWT) in patients with facioscapulohumeral muscular dystrophy (FSHD).
Genetically defined and clinically affected FSHD participants at 2 sites performed the 6MWT, the Timed Up and Go, and the 30 foot Go/Timed 10 meter test as measures of mobility using standard procedures.
Eight-six participants representing the full range of severity performed the 6MWT. The mean 6MWT distance was 404.3 meters (SD 123.9), with no difference between sites. The 6MWT was reliable (n = 25; intraclass correlation coefficient = 0.99) and demonstrated moderate to strong correlations with lower extremity strength, functional outcomes, and FSHD Clinical Score.
The 6MWT is reliable and is associated with other measures of FSHD disease severity. Future directions include assessing its sensitivity to disease progression. Muscle Nerve 55: 333-337, 2017.
为未来的临床试验做准备,我们确定了面肩肱型肌营养不良症(FSHD)患者6分钟步行试验(6MWT)的可靠性、与疾病严重程度测量指标的关系以及不同研究地点之间的一致性。
两个研究地点的经基因定义且有临床症状的FSHD参与者按照标准程序进行6MWT、定时起立行走试验以及30英尺行走/定时10米试验,以评估其活动能力。
86名代表不同严重程度的参与者进行了6MWT。6MWT的平均距离为404.3米(标准差123.9),不同研究地点之间无差异。6MWT具有可靠性(n = 25;组内相关系数 = 0.99),并且与下肢力量、功能结局以及FSHD临床评分呈中度到高度相关。
6MWT具有可靠性,并且与FSHD疾病严重程度的其他测量指标相关。未来的研究方向包括评估其对疾病进展的敏感性。《肌肉与神经》55: 333 - 337, 2017年。